Intestinal Transplantation in Children with Intestinal Failure

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Chapter 331 Intestinal Transplantation in Children with Intestinal Failure

Jorge D. Reyes

The introduction of tacrolimus and the development of the abdominal multiorgan procurement techniques allowed the tailoring of various types of intestine grafts, which can contain other intra-abdominal organs such as the liver, pancreas, and stomach; this tailoring has been critical to the application of this type of organ transplant, given the wide scope of diseases for which replacement of the intestine may be necessary. Also, the understanding that the liver protects the intestine against rejection had been suggested by previous combinations of liver plus other organs such as the kidney. The first survivors of intestine transplantation would also go on to demonstrate the interaction (host-versus-graft and graft-versus-host) between recipient and donor immunocytes (brought with the allograft), which under the cover of immunosuppression allows varying degrees of graft acceptance and eventual minimization of drug therapy.

Indications for Intestinal Transplant

Intestinal failure (IF) describes a patient who has lost the ability to maintain nutritional support with his or her intestine and is permanently dependent on total parenteral nutrition (TPN). The majority of these patients have short bowels as a result of a congenital deficiency or acquired condition (Chapter 330.7). In others, the cause of IF is a functional disorder of motility or absorption (Table 331-1). Rarely do patients receive intestinal transplants for benign neoplasms. IF is a syndrome that includes “satellite” complications such as loss of venous access, life-threatening infections, and TPN-induced cholestatic liver disease. Patients who develop these complications have a ∼70% 1 yr mortality and thus require organ replacement therapy with intestinal transplantation.

Table 331-1 CAUSES OF INTESTINAL FAILURE REQUIRING TRANSPLANTATION

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Paucity of Venous Access

Administration of TPN requires the insertion of a centrally placed venous catheter, and there are only 6 readily accessible sites (bilateral internal jugulars, subclavians, and iliac veins). The loss of venous access generally occurs in the setting of recurrent catheter sepsis and thrombosis; loss of 50% of these venous access sites places the patient at risk of not being able to be treated with TPN.

Life-Threatening Infections

Life-threatening infections are usually catheter-related; the absence of significant lengths of intestine may be associated with abnormal motility of the residual bowel (producing both delayed or rapid emptying), with varying degrees of bacterial overgrowth and possible bacterial translocation as a consequence of loss of intestinal barrier function and/or loss of gut immunity. This situation can produce cholestatic liver disease, multisystem organ failure, and metastatic infectious foci in lungs, kidneys, liver, and the brain.

Liver Disease

The development of cholestatic liver disease may be a consequence of the toxic drug effects of TPN on hepatocytes, a disruption of bile flow and bile acid metabolism, and the frequent occurrence of bacterial translocation and sepsis with endotoxin release into the portal circulation. This complication varies in frequency depending on age and the etiology of the IF; it is most common in neonates with extremely short gut. The effects on the liver include fatty transformation, steatohepatitis and necrosis, fibrosis, and then cholestasis. Development of clinical jaundice (total bilirubin >3 mg/dL) and thrombocytopenia are significant risk factors for poor outcome, because these changes lead to the development of portal hypertensive gastroenteropathy, hypersplenism, coagulopathy, and uncontrollable bleeding.

Transplant Operation

Donor Selection

Intestinal grafts are usually procured from hemodynamically stable, ABO-identical brain-dead donors who have minimal clinical or laboratory evidence suggesting intra-abdominal ischemia. Human leukocyte antigen (HLA) has been random, and cytomegalovirus-positive donors have been avoided in recipients of isolated intestine grafts. Exclusion criteria include a history of malignancy and intra-abdominal evidence of infection; systemic viral or bacterial infections are not excluded. Donor preparation has been limited to the administration of systemic and enteral antibiotics. Prophylaxis for graft versus host disease (GVHD) with graft pretreatment using irradiation or a monoclonal antilymphocyte antibody has varied over time and among the centers performing this procedure. Grafts have been preserved with the University of Wisconsin solution.

Types of Intestinal Grafts

Intestinal allografts are used in various forms, either alone (as an isolated intestine graft) or as a composite graft, which can include the liver, duodenum, and pancreas (liver-intestine graft); when this composite graft includes the stomach, and the recipient operation requires the replacement of all of the patient’s gastrointestinal tract (as with intestinal pseudo-obstruction) and liver, then this replacement graft is known as a multivisceral graft.

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